Skip to main content

Advertisement

SpringerLink
Log in

Treatment of refractory polymyositis and dermatomyositis

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Polymyositis (PM) and dermatomyositis (DM) are autoimmune inflammatory diseases that primarily target muscle. Although similar, PM and DM have different pathophysiologic mechanisms. Current therapy for PM and DM does not take into account these pathophysiologic differences. Recent work has started to define outcome measures to apply to future therapeutic trials, which will allow better comparison of treatment outcomes. For patients who are unresponsive to standard therapy with high dose prednisone supplemented by methotrexate and/or azathioprine it is not clear what represents the next best choice for therapy. Although there are few controlled studies in the area, there is reason to be optimistic for use of intravenous methylprednisolone pulses, intravenous gammaglobulin, cyclosporine A, tacrolimus, mycophenolate mofetil, and rituximab for nonresponding patients. Better understanding of the underlying pathophysiology of PM and DM, as well as carefully performed multicenter clinical trials is going to be necessary to make better recommendations in the future.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Dalakas MC, Hohlfeld R: Polymyositis and dermatomyositis. Lancet 2003, 362:971–982.

    Article  PubMed  CAS  Google Scholar 

  2. Arahata K, Engel AG: Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells. Ann Neurol 1984, 16:193–208.

    Article  PubMed  CAS  Google Scholar 

  3. Miller FW, Rider LG, Chung YL, et al.: Proposed preliminary core set measures for disease outcome assessment in adult and juvenile idiopathic inflammatory myopathies. Rheumatology (Oxford) 2001, 40:1262–1273.

    Article  CAS  Google Scholar 

  4. Walton J, Adams R: Polymyositis. Edinburgh: E & S Livingstone Ltd.; 1958.

    Google Scholar 

  5. Winkelmann RK, Mulder DW, Lambert EH, et al.: Course of dermatomyositis-polymyositis: comparison of untreated and cortisone-treated patients. Mayo Clin Proc 1968, 43:545–556.

    PubMed  CAS  Google Scholar 

  6. Lundberg I, Kratz AK, Alexanderson H, Patarroyo M: Decreased expression of interleukin-1alpha, interleukin-1beta, and cell adhesion molecules in muscle tissue following corticosteroid treatment in patients with polymyositis and dermatomyositis. Arthritis Rheum 2000, 43:336–348.

    Article  PubMed  CAS  Google Scholar 

  7. Ponyi A, Borgulya G, Constantin T, et al.: Functional outcome and quality of life in adult patients with idiopathic inflammatory myositis. Rheumatology (Oxford) 2005, 44:83–88.

    Article  CAS  Google Scholar 

  8. Choy EH, Hoogendijk JE, Lecky B, Winer JB: Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev 2005, CD003643.This systematic review documents the sad state of science as it relates to formal studies of PM and DM.

  9. Dalakas MC, Illa I, Dambrosia JM, et al.: A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993, 329:1993–2000.

    Article  PubMed  CAS  Google Scholar 

  10. Vencovsky J, Jarosova K, Machacek S, et al.: Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis. Scand J Rheumatol 2000, 29:95–102.

    Article  PubMed  CAS  Google Scholar 

  11. Bunch TW, Worthington JW, Combs JJ, et al.: Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med 1980, 92:365–369.

    PubMed  CAS  Google Scholar 

  12. Bunch TW: Prednisone and azathioprine for polymyositis: long-term followup. Arthritis Rheum 1981, 24:45–48.

    Article  PubMed  CAS  Google Scholar 

  13. Metzger AL, Bohan A, Goldberg LS, et al.: Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy. Ann Intern Med 1974, 81:182–189.

    PubMed  CAS  Google Scholar 

  14. Villalba L, Hicks JE, Adams EM, et al.: Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 1998, 41:392–399.

    Article  PubMed  CAS  Google Scholar 

  15. Miller FW, Leitman SF, Cronin ME, et al.: Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992, 326:1380–1384.

    Article  PubMed  CAS  Google Scholar 

  16. Huang JL: Long-term prognosis of patients with juvenile dermatomyositis initially treated with intravenous methylprednisolone pulse therapy. Clin Exp Rheumatol 1999, 17:621–624.

    PubMed  CAS  Google Scholar 

  17. Al-Mayouf S, Al-Mazyed A, Bahabri S: Efficacy of early treatment of severe juvenile dermatomyositis with intravenous methylprednisolone and methotrexate. Clin Rheumatol 2000, 19:138–141.

    Article  PubMed  CAS  Google Scholar 

  18. Lang B, Dooley J: Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis. J Pediatr 1996, 128:429–432.

    Article  PubMed  CAS  Google Scholar 

  19. Bolosiu HD, Man L, Rednic S: The effect of methylprednisolone pulse therapy in polymyositis/dermatomyositis. Adv Exp Med Biol 1999, 455:349–357.

    PubMed  CAS  Google Scholar 

  20. Huber AM, Lang B, LeBlanc CM, et al.: Medium- and longterm functional outcomes in a multicenter cohort of children with juvenile dermatomyositis. Arthritis Rheum 2000, 43:541–549.

    Article  PubMed  CAS  Google Scholar 

  21. Al-Mayouf SM, Laxer RM, Schneider R, et al.: Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety. J Rheumatol 2000, 27:2498–2503.

    PubMed  CAS  Google Scholar 

  22. Gottfried I, Seeber A, Anegg B, et al.: High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels. Eur J Dermatol 2000, 10:29–35.

    PubMed  CAS  Google Scholar 

  23. Cherin P, Pelletier S, Teixeira A, et al.: Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. Arthritis Rheum 2002, 46:467–474. An open-label study describing treatment of PM with IVIg. Seventy percent of patients responded.

    Article  PubMed  CAS  Google Scholar 

  24. Zeller V, Cohen P, Prieur AM, Guillevin L: Cyclosporin a therapy in refractory juvenile dermatomyositis. Experience and longterm followup of 6 cases. J Rheumatol 1996, 23:1424–1427.

    PubMed  CAS  Google Scholar 

  25. Maeda K, Kimura R, Komuta K, Igarashi T: Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis? Scand J Rheumatol 1997, 26:24–29.

    Article  PubMed  CAS  Google Scholar 

  26. Qushmaq KA, Chalmers A, Esdaile JM: Cyclosporin A in the treatment of refractory adult polymyositis/dermatomyositis: population based experience in 6 patients and literature review. J Rheumatol 2000, 27:2855–2859.

    PubMed  CAS  Google Scholar 

  27. Nawata Y, Kurasawa K, Takabayashi K, et al.: Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol 1999, 26:1527–1533.

    PubMed  CAS  Google Scholar 

  28. Fujisawa T, Suda T, Nakamura Y, et al.: Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol 2005, 32:58–64. This report focuses on ILD and details responses with various therapies. Patients with ILD associated with DM had a poorer prognosis than those associated with PM.

    PubMed  Google Scholar 

  29. Oddis CV, Sciurba FC, Elmagd KA, Starzl TE: Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet 1999, 353:1762–1763.

    Article  PubMed  CAS  Google Scholar 

  30. Ochi S, Nanki T, Takada K, et al.: Favorable outcomes with tacrolimus in two patients with refractory interstitial lung disease associated with polymyositis/dermatomyositis. Clin Exp Rheumatol 2005, 23:707–710.

    PubMed  CAS  Google Scholar 

  31. Takada K, Nagasaka K, Miyasaka N: Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants. Autoimmunity 2005, 38:383–392. Retrospective review of ILD associated with PM and DM. Treatment with steroids and CSA was effective for most patients other than those with DM and acute ILD.

    Article  PubMed  CAS  Google Scholar 

  32. Yoshimasu T, Ohtani T, Sakamoto T, et al.: Topical FK506 (tacrolimus) therapy for facial erythematous lesions of cutaneous lupus erythematosus and dermatomyositis. Eur J Dermatol 2002, 12:50–52.

    PubMed  Google Scholar 

  33. Garcia-Doval I, Cruces M: Topical tacrolimus in cutaneous lesions of dermatomyositis: lack of effect in side-by-side comparison in five patients. Dermatology 2004, 209:247–248.

    Article  PubMed  CAS  Google Scholar 

  34. Gelber AC, Nousari HC, Wigley FM: Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. J Rheumatol 2000, 27:1542–1545.

    PubMed  CAS  Google Scholar 

  35. Majithia V, Harisdangkul V: Mycophenolate mofetil (Cell- Cept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford) 2005, 44:386–389.

    Article  CAS  Google Scholar 

  36. Edge JC, Outland JD, Dempsey JR, Callen JP: Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Arch Dermatol 2006, 142:65–69. Open-label treatment of patients with recalcitrant cutaneous lesions of DM treated with MMF. MMF is helpful but slow to act.

    Article  PubMed  CAS  Google Scholar 

  37. Gottenberg JE, Guillevin L, Lambotte O, et al.: Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases. Ann Rheum Dis 2005, 64:913–920.

    Article  PubMed  CAS  Google Scholar 

  38. Chiappetta N, Steier J, Gruber B: Rituximab in the treatment of refractory dermatomyositis. J Clin Rheumatol 2005, 11:264–266.

    Article  PubMed  Google Scholar 

  39. Lambotte O, Kotb R, Maigne G, et al.: Efficacy of rituximab in refractory polymyositis. J Rheumatol 2005, 32:1369–1370.

    PubMed  Google Scholar 

  40. Levine TD: Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum 2005, 52:601–607. A small open-label study demonstrating the value of rituximab for therapy of refractory DM.

    Article  PubMed  CAS  Google Scholar 

  41. Edwards JC, Szczepanski L, Szechinski J, et al.: Efficacy of B-cell-targeted therapy with rituximab in patients with rheumatoid arthritis. N Engl J Med 2004, 350:2572–2581.

    Article  PubMed  CAS  Google Scholar 

  42. Riley P, Maillard SM, Wedderburn LR, et al.: Intravenous cyclophosphamide pulse therapy in juvenile dermatomyositis. A review of efficacy and safety. Rheumatology (Oxford) 2004, 43:491–496. This important study demonstrates the value of cyclophosphamide in severe JDM. In extreme situations, extreme measures are warranted. Unfortunately, calcinosis was unchanged.

    Article  CAS  Google Scholar 

  43. Hengstman GJ, van den Hoogen FH, van Engelen BG: Treatment of dermatomyositis and polymyositis with anti-tumor necrosis factor-alpha: long-term follow-up. Eur Neurol 2004, 52:61–63.

    Article  PubMed  CAS  Google Scholar 

  44. Labioche I, Liozon E, Weschler B, et al.: Refractory polymyositis responding to infliximab: extended follow-up. Rheumatology (Oxford) 2004, 43:531–532.

    Article  CAS  Google Scholar 

  45. Uthman I, El-Sayad J: Refractory polymyositis responding to infliximab. Rheumatology (Oxford) 2004, 43:1198–1199.

    Article  CAS  Google Scholar 

  46. Sprott H, Glatzel M, Michel BA: Treatment of myositis with etanercept (Enbrel), a recombinant human soluble fusion protein of TNF-alpha type II receptor and IgG1. Rheumatology (Oxford) 2004, 43:524–526.

    Article  CAS  Google Scholar 

  47. Marie I, Heliot P, Roussel F, et al.: Fatal Mycobacterium peregrinum pneumonia in refractory polymyositis treated with infliximab. Rheumatology (Oxford) 2005, 44:1201–1202.

    Article  CAS  Google Scholar 

  48. Kameda H, Nagasawa H, Ogawa H, et al.: Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 2005, 32:1719–1726.

    PubMed  CAS  Google Scholar 

  49. Adams EM, Pucino F, Yarboro C, et al.: A pilot study: use of.udarabine for refractory dermatomyositis and polymyositis, and examination of endpoint measures. J Rheumatol 1999, 26:352–360.

    PubMed  CAS  Google Scholar 

  50. Nagaraju K, Raben N, Villalba ML, et al.: Costimulatory markers in muscle of patients with idiopathic inflammatory myopathies and in cultured muscle cells. Clin Immunol 1999, 92:161–169.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street SW, 55905, Rochester, MN, USA

    Steven R. Ytterberg MD

Authors
  1. Steven R. Ytterberg MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Steven R. Ytterberg MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ytterberg, S.R. Treatment of refractory polymyositis and dermatomyositis. Curr Rheumatol Rep 8, 167–173 (2006). https://doi.org/10.1007/s11926-996-0021-7

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-996-0021-7

Keywords

Search

Navigation